Treatment Options for Hypertrophic Cardiomyopathy (HCM)

Medical Management

Beta-blockers are the cornerstone of medical therapy for symptomatic HCM patients with either obstructive or non-obstructive disease, targeting a resting heart rate of <60-65 bpm 1
Beta-blockers provide symptomatic relief through negative inotropic effects and by attenuating adrenergic-induced tachycardia, which prolongs diastolic filling period 2
For patients unable to tolerate beta-blockers, non-dihydropyridine calcium channel blockers (verapamil or diltiazem) may be used as alternative first-line agents 1, 2
Verapamil should be used cautiously in patients with severe outflow tract obstruction, elevated pulmonary pressures, or low systemic blood pressure, as it may trigger increased obstruction and pulmonary edema 3

For patients with persistent symptoms despite beta-blockers or calcium channel blockers, disopyramide may be added (in combination with beta-blockers or verapamil) 2, 1
Disopyramide provides additional negative inotropic effects that can reduce outflow tract obstruction 1
Diuretics may be cautiously added for patients with persistent congestive symptoms, but should be used carefully to avoid excessive preload reduction 2, 1
Cardiac myosin inhibitors (mavacamten) are now available for patients with symptomatic obstructive HCM who don't achieve adequate relief from first-line therapies 2

Dihydropyridine calcium channel blockers (e.g., nifedipine) should be avoided in patients with obstructive HCM due to their vasodilatory effects that may worsen obstruction 1, 2
Vasodilators such as ACE inhibitors and ARBs should be used cautiously or avoided in obstructive HCM 1
Mavacamten is contraindicated during pregnancy due to potential teratogenic effects 2

Septal reduction therapy should be considered for severely symptomatic patients with LVOT obstruction (gradient ≥50 mmHg) who remain symptomatic despite optimal medical therapy 2, 1
Two main options exist: surgical septal myectomy and alcohol septal ablation 2
These procedures should be performed at experienced HCM centers with dedicated teams for optimal outcomes 2

Surgical myectomy involves direct resection of the hypertrophied septal muscle to relieve obstruction 2
Transesophageal echocardiography is recommended for intraoperative guidance during surgical myectomy 2
Post-procedure TTE should be used to evaluate the effects of surgical myectomy 2

Alcohol septal ablation is a percutaneous alternative to surgery that creates a controlled infarct in the basal septum 2
TTE or TEE with intracoronary contrast injection is recommended for intraprocedural guidance 2
Post-procedure TTE should be used to evaluate the effects of alcohol septal ablation 2

Anticoagulation with vitamin K antagonists or direct oral anticoagulants is indicated in all HCM patients with atrial fibrillation, regardless of CHA₂DS₂-VASc score 2, 1
Ventricular rate control in HCM patients with AF may require high doses of beta-blockers and non-dihydropyridine calcium channel blockers 2

Risk stratification for sudden cardiac death should be performed in all HCM patients, regardless of symptoms 2
Implantable cardioverter-defibrillator (ICD) placement decisions should incorporate established risk markers and individual risk scores 2
Risk factors include family history of sudden death, LV wall thickness ≥30 mm, unexplained syncope, nonsustained ventricular tachycardia, and abnormal blood pressure response to exercise 2

Low-intensity competitive sports (e.g., golf, bowling) are reasonable for HCM patients 2
For most patients with HCM, universal restriction from vigorous physical activity is not indicated 2
Participation in vigorous recreational activities is reasonable after comprehensive evaluation and shared decision-making with an HCM expert 2
Participation in high-intensity competitive sports may be considered in selected cases after thorough evaluation 2

Combining verapamil with beta-blockers requires caution due to potential for high-grade AV block and excessive bradycardia 3
Verapamil should be avoided in patients with severe left ventricular dysfunction (ejection fraction <30%) 3
In patients with hypertrophic cardiomyopathy, verapamil can cause serious adverse effects including pulmonary edema and severe hypotension, particularly in those with severe outflow obstruction 3
Septal reduction therapy should not be performed in asymptomatic patients regardless of gradient severity 1